Ignite Creation Date:
2024-10-26 @ 3:39 PM
Last Modification Date:
2024-10-26 @ 3:39 PM
Study NCT ID:
NCT06583304
Status:
NOT_YET_RECRUITING
Last Update Posted:
None
First Post:
2024-09-01
Brief Title:
Hematological Indices in Pediatric Diagnosed with Familial Mediterranean Fever
Sponsor:
None
Organization:
None
Study Overview
Official Title:
Clinical Criteria and Hematological Indices in Pediatrics Diagnosed with Familial Mediterranean Fever a Single Center Study in Upper Egypt
Status:
NOT_YET_RECRUITING
Status Verified Date:
2024-09
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
No
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
Brief Summary:
To investigate the relationship between the hematological indices and subclinical inflammation in FMF patients during their attack-free period additionally this study aims to assess the potential of these indices as predictive markers for ongoing inflammation in these patients population at Assuit university hospital of pediatrics
Detailed Description:
Familial Mediterranean fever FMF is the most common hereditary auto inflammatory disease in the world FMF is caused by gain of function mutations in the MEFV gene which encodes a protein called pyrin which has regulatory functions on the innate immune system 1 Mutations interfere with the function of the pyrin domain initiating an uninterrupted inflammatory cascade 2 Familial Mediterranean fever affects the populations located on the Mediterranean basin It is virtually limited to Armenians Turks Arabs and nonAshkenazi Jews However some cases have been described in European countries including Italy France Spain Portugal and Greece 3 The clinical presentation of FMF can be variable likely depending on its genetic heterogeneity and environmental factors However the clinical picture is usually very suggestive of the underlying disease It is typically characterized by recurrent episodes of fever and systemic inflammation with pleural and peritoneal serositis and arthritis Starting in childhood patients present shortlasting self-resolving attacks of fever and abdominal chest or joint pain with systemic inflammation Periodicity is not strict and episodes may occur from once a week up to once every three to four months or more in untreated patients These events are usually very disabling and in clear contrast with complete well-being in attack-free periods 4 The diagnosis of FMF based on clinical findings is supported by ethnic origin family history and genetic testing Several criteria had been developed for the diagnosis of FMF the most widely used Tel-Hashomer criteria Most recently adult and pediatric rheumatology experts have created a new set of classifcation criteria EurofeverPRINTO classifcation criteria 5
During FMF attacks many acute phase reactants such as C-reactive protein CRP serum amyloid A fibrinogen and erythrocyte sedimentation rate ESR increase It is known that a subclinical inflammation continues in FMF patients during the inter attack period and this increases the risk of developing amyloidosis 6 Therefore the use of additional serum markers to evaluate disease activity may provide beneficial advantages for monitoring and preventing further complications Systemic inflammation also causes changes in the number and composition of circulating blood cells 7 8 Neutrophils lymphocytes and platelets play major roles in systemic inflammation 9 Neutrophils and platelets secrete proinflammatory cytokines that augment of more neutrophils and platelets 10 Microparticles released from activated platelets interact with neutrophils through platelet-like lipoxygenase expression and activation of eicosanoid pathway 11 The platelet count mean platelet volume MPV are the most important indices of platelet function and activation MPV a component of routine complete blood count CBC tests is inversely affected by systemic inflammation MPV levels were shown to be lower in active inflammatory bowel diseases rheumatoid arthritis RA and ankylosing spondylitis AS and increased MPV values were reported after treatment Additionally the neutrophil-to-lymphocyte ratio NLR and the platelet-to-lymphocyte ratio PLR which are easily obtained from differential CBC have been shown to not only increase in inflammation but also be correlated with disease activity and prognosis 12 We discuss the clinical criteria and the association of hematological indices with subclinical inflammation and to evaluate their usability as predictors of ongoing inflammation in FMF patients during their attack-free period
During FMF attacks many acute phase reactants such as C-reactive protein CRP serum amyloid A fibrinogen and erythrocyte sedimentation rate ESR increase It is known that a subclinical inflammation continues in FMF patients during the inter attack period and this increases the risk of developing amyloidosis 6 Therefore the use of additional serum markers to evaluate disease activity may provide beneficial advantages for monitoring and preventing further complications Systemic inflammation also causes changes in the number and composition of circulating blood cells 7 8 Neutrophils lymphocytes and platelets play major roles in systemic inflammation 9 Neutrophils and platelets secrete proinflammatory cytokines that augment of more neutrophils and platelets 10 Microparticles released from activated platelets interact with neutrophils through platelet-like lipoxygenase expression and activation of eicosanoid pathway 11 The platelet count mean platelet volume MPV are the most important indices of platelet function and activation MPV a component of routine complete blood count CBC tests is inversely affected by systemic inflammation MPV levels were shown to be lower in active inflammatory bowel diseases rheumatoid arthritis RA and ankylosing spondylitis AS and increased MPV values were reported after treatment Additionally the neutrophil-to-lymphocyte ratio NLR and the platelet-to-lymphocyte ratio PLR which are easily obtained from differential CBC have been shown to not only increase in inflammation but also be correlated with disease activity and prognosis 12 We discuss the clinical criteria and the association of hematological indices with subclinical inflammation and to evaluate their usability as predictors of ongoing inflammation in FMF patients during their attack-free period
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
None
Is a FDA Regulated Device?:
None
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None